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MGP000660

UniProt Annotations

Entry Information
Gene Nameceruloplasmin (ferroxidase)
Protein EntryCERU_HUMAN
UniProt IDP00450
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity4 Fe(2+) + 4 H(+) + O(2) = 4 Fe(3+) + 2 H(2)O.
CofactorName=Cu cation; Xref=ChEBI:CHEBI:23378; Note=Binds 6 Cu cations per monomer.;
DiseaseAceruloplasminemia (ACERULOP) [MIM:604290]: An autosomal recessive disorder of iron metabolism characterized by iron accumulation in the brain as well as visceral organs. Clinical features consist of the triad of retinal degeneration, diabetes mellitus and neurological disturbances. Note=The disease is caused by mutations affecting the gene represented in this entry.
DiseaseNote=Ceruloplasmin levels are decreased in Wilson disease, in which copper cannot be incorporated into ceruloplasmin in liver because of defects in the copper-transporting ATPase 2.
FunctionCeruloplasmin is a blue, copper-binding (6-7 atoms per molecule) glycoprotein. It has ferroxidase activity oxidizing Fe(2+) to Fe(3+) without releasing radical oxygen species. It is involved in iron transport across the cell membrane. Provides Cu(2+) ions for the ascorbate-mediated deaminase degradation of the heparan sulfate chains of GPC1. May also play a role in fetal lung development or pulmonary antioxidant defense (By similarity). {ECO:0000250}.
SimilarityBelongs to the multicopper oxidase family. {ECO:0000305}.
SimilarityContains 3 F5/8 type A domains. {ECO:0000305}.
SimilarityContains 6 plastocyanin-like domains. {ECO:0000305}.
Subcellular LocationSecreted. Note=Colocalizes with GCP1 in secretory intracellular compartments. {ECO:0000250}.
Tissue SpecificityExpressed by the liver and secreted in plasma.
Web ResourceName=Wikipedia; Note=Ceruloplasmin entry; URL="http://en.wikipedia.org/wiki/Ceruloplasmin";
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